The Colorful Palette of Neuroendocrine Neoplasms in the Genitourinary Tract

Pósfai, Boglárka; Kuthi, Levente; Varga, Linda; Laczó, Ibolya; Révész, János; Kránicz, Réka; Maráz, Anikó

doi:10.21873/anticanres.12589

Cited by 18 publications

(27 citation statements)

References 64 publications

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“…Typical symptoms of the carcinoid syndrome, including flushing, edema, and diarrhea, are uncommon and are seen in <10-15% of patients [5,[10][11][12]. The incidence of small-cell renal NEC is rare; approximately 50 cases have been reported in the literature [13][14][15][16][17]. Most of the patients present at the age of 59 years with non-specific symptoms, such as hematuria, weight loss, and abdominal pain.…”

Section: Epidemiology Presentation and Pathogenesismentioning

confidence: 99%

Neuroendocrine Neoplasms of the Female Genitourinary Tract: A Comprehensive Overview

Virarkar

Vulasala

Gopireddy

et al. 2022

Cancers

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Primary neuroendocrine neoplasms are a rare heterogeneous group of tumors that include well-differentiated neuroendocrine tumors, poorly differentiated neuroendocrine carcinoma, and paraganglioma. An extensive literature search was used to compile the data regarding epidemiology, pathogenesis, imaging features, and management of the urinary system NENs. We also included the updated staging of the NENs at various locations of the urinary system.

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Section: Epidemiology Presentation and Pathogenesismentioning

confidence: 99%

Neuroendocrine Neoplasms of the Female Genitourinary Tract: A Comprehensive Overview

Virarkar

Vulasala

Gopireddy

et al. 2022

Cancers

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“…Like pulmonary small cell carcinoma, SCCUT cells have high rates of proliferation (4). Patients typically present with aggressive tumors, commonly muscle invasive, and there is association with other aggressive variants of urothelial carcinoma (UC) (2,5). Five-year survival for patients diagnosed with SCCUT is 16-25% (compared to 77% in pure UC) likely due to the metastatic and the heterogeneous nature of this disease (2,6).…”

Section: Introductionmentioning

confidence: 99%

p53 Expression, Programmed Death Ligand 1, and Risk Factors in Urinary Tract Small Cell Carcinoma

et al. 2021

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Introduction: Small cell carcinoma of the urinary tract (SCCUT) is a rare finding with poor clinical course. This study sheds light on the molecular subtype and identifies risk factors in patients diagnosed with SCCUT.Methods: Immunohistochemical expression of immunotherapy target programmed death ligand 1 (PD-L1) and luminal (GATA3), basal (p63), and p53 markers are assessed in patients diagnosed with SCCUT. Univariate analysis identified risk factors. Overall survival (OS) is computed using the Kaplan–Meier method.Results: Tissue was available for 70.2% (33/47). All showed a high PD-L1 expression phenotype. p53 is seen in 93.9% (31/33), mostly as overexpression, GATA3 in 45.5% (15/33), and p63 in 57.6% (19/33). For the entire cohort (n = 47), 1-year survival was 59.6%, and the median OS was 17 months. Univariate analysis shows that chemotherapy [hazard ratio (HR) = 0.29, 95% confidence interval (CI) = 0.14–0.61, p = 0.001], radical surgery (HR = 0.37, 95% CI = 0.18–0.76, p = 0.007), and diagnosis of non-pure SCCUT (HR = 0.44, 95% CI = 0.22–0.86, p = 0.02) are favorable prognostic features. Metastasis had negative associations with survival (HR = 2.1, 95% CI = 1.1–4.2, p = 0.03).Conclusions: In this series, pure and mixed SCCUT are characterized by p53 overexpression and a high PD-L1 phenotype. Histology of non-pure SCCUT is a positive prognosticator, and radical cystectomy or chemotherapy can improve OS. These findings demonstrate that SCCUT may be eligible for PD-L1 immunotherapy.

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“…Poorly differentiated neuroendocrine carcinoma (NEC) of the bladder (BNEC) is a rare type of bladder cancer, summing up to less than 1% of bladder malignancies [1]. The neuroendocrine component can be found either alone, mostly with small-cell or large-cell morphology, or can be mixed to an epithelial component (mixed adeno-neuroendocrine carcinoma, MANEC), that is usually a urothelial carcinoma [1]. Some features of BNEC are shared with small-cell carcinoma of the lung, such as pathology [2] and the typical genomic alterations, TP53 and RB1 mutation or loss [3].…”

Section: Introductionmentioning

confidence: 99%

Perioperative Chemotherapy in Poorly Differentiated Neuroendocrine Neoplasia of the Bladder: A Multicenter Analysis

Brizzi

Pusceddu

Gelsomino

et al. 2020

JCM

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There is scant evidence about optimal management of poorly differentiated neuroendocrine carcinoma of the bladder (BNEC). We performed a multicenter retrospective study on BNEC patients from 13 Italian neuroendocrine-dedicated centers to analyze strategies associated with better outcomes. Mixed adeno-neuroendocrine carcinomas (MANEC) were included. We analyzed overall survival (OS) in the overall cohort, relapse-free survival (RFS) in radically operated patients and progression-free survival (PFS) in patients who received chemotherapy for metastatic disease. Fifty-one BNEC patients were included (male: 46, median age: 70 years). Overall, median OS was 16.0 months, radical tumor resection was performed in 37 patients (72.5%) and 11 of these (29.7%) also received peri-operative platinum-etoposide chemotherapy. Median OS was longer in patients with better performance status (PS) and in those with stage I–III disease at diagnosis compared to stage IV. Among patients who underwent radical tumor resection (N = 37), RFS was longer in patients with better PS and showed a trend towards a longer RFS in those treated with peri-operative chemotherapy than with surgery alone (11 vs. 6 months; p = 0.078). Among 28 patients receiving chemotherapy for metastatic disease, PFS was 5.0 months and there was a trend towards improved PFS in patients receiving carboplatin-etoposide chemotherapy compared to other regimens. A multivariate model unmasked a significant association between carboplatin-etoposide chemotherapy and risk for disease progression or death (HR: 0.39 (95%CI: 0.16–0.96) p = 0.040). Performance status might be associated with improved RFS in radically operated patients, while type of chemotherapy might affect PFS in patients receiving chemotherapy for metastatic BNEC.

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The Colorful Palette of Neuroendocrine Neoplasms in the Genitourinary Tract

Cited by 18 publications

References 64 publications

Neuroendocrine Neoplasms of the Female Genitourinary Tract: A Comprehensive Overview

Neuroendocrine Neoplasms of the Female Genitourinary Tract: A Comprehensive Overview

p53 Expression, Programmed Death Ligand 1, and Risk Factors in Urinary Tract Small Cell Carcinoma

Perioperative Chemotherapy in Poorly Differentiated Neuroendocrine Neoplasia of the Bladder: A Multicenter Analysis

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